Ramos-Casals, M., Brito-Zeron, P., Bombardieri, S., Bootsma, H., De Vita, S., Dorner, T., Fisher, B. A., Gottenberg, J. -E., Hernandez-Molina, G., Kocher, A., Kostov, B., Kruize, A. A., Mandl, T., W. -F., Ng, Retamozo, S., Seror, R., Shoenfeld, Y., Siso-Almirall, A., Tzioufas, A. G., Vitali, C., Bowman, S., EULAR-Sjögren Syndrome Task Force Group: Sebastian A, Mariette X., Saraux, A, Vissink, A, Rasmussen, A, Hofauer, B, Armagan, B, Feijoo-Massó, C, Shiboski, Ch, Baldini, C, Vollenweider, C, Sene, D, Hammenfors, D, Isenberg, D, Danda, D, Bartoloni, E, Hospital Clinic i Provincial de Barcelona (SCReN), CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, H. CIMA-Sanitas, Barcelona, University of Pisa - Università di Pisa, University of Groningen [Groningen], Ospedale 'Santa Maria della Misericordia' = University Hospital 'Santa Maria della Misericordia', Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], University of Birmingham [Birmingham], Service de rhumatologie [Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Immunopathologie et chimie thérapeutique (ICT), Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán - National Institute of Medical Science and Nutrition Salvador Zubiran [Mexico], University Hospital Basel [Basel], Bern University Hospital [Berne] (Inselspital), Transverse group for research in primary care [Barcelona], Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat Politècnica de Catalunya [Barcelona] (UPC), University Medical Center [Utrecht], Skane University Hospital [Malmo], Lund University [Lund], Newcastle University [Newcastle], Newcastle Upon Tyne Hospitals NHS Foundation Trust, Universidad de Córdoba = University of Córdoba [Córdoba], Universidad Nacional de Córdoba [Argentina], Immunologie des Maladies Virales et Autoimmunes (IMVA - U1184), Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), The Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Saint Petersburg University (SPBU), CAP Les Corts (CAPSBE), National and Kapodistrian University of Athens (NKUA), Santo Stefano Riabilitazione, University Hospitals Birmingham [Birmingham, Royaume-Uni], Centre National de la Recherche Scientifique (CNRS)-Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), Rheumatology Unit, Cordoba (Institute University of Biomedical Sciences University of Cordoba (IUCBC), Universitat Politècnica de Catalunya. Departament d'Estadística i Investigació Operativa, and Salvy-Córdoba, Nathalie
International audience; The therapeutic management of Sjögren syndrome (SjS) has not changed substantially in recent decades: treatment decisions remain challenging in clinical practice, without a specific therapeutic target beyond the relief of symptoms as the most important goal. In view of this scenario, the European League Against Rheumatism (EULAR) promoted and supported an international collaborative study (EULAR SS Task Force) aimed at developing the first EULAR evidence and consensus-based recommendations for the management of patients with SjS with topical and systemic medications. The aim was to develop a rational therapeutic approach to SjS patients useful for healthcare professionals, physicians undergoing specialist training, medical students, the pharmaceutical industry and drug regulatory organisations following the 2014 EULAR standardised operating procedures. The Task Force (TF) included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, statisticians, general practitioners, nurses and patient representatives from 30 countries of the 5 continents. Evidence was collected from studies including primary SjS patients fulfilling the 2002/2016 criteria; when no evidence was available, evidence from studies including associated SjS or patients fulfilling previous sets of criteria was considered and extrapolated. The TF endorsed the presentation of general principles for the management of patients with SjS as three overarching, general consensus-based recommendations and 12 specific recommendations that form a logical sequence, starting with the management of the central triplet of symptoms (dryness, fatigue and pain) followed by the management of systemic disease. The recommendations address the use of topical oral (saliva substitutes) and ocular (artificial tear drops, topical non-steroidal anti-inflammatory drugs, topical corticosteroids, topical CyA, serum tear drops) therapies, oral muscarinic agonists (pilocarpine, cevimeline), hydroxychloroquine, oral glucocorticoids, synthetic immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, leflunomide and mycophenolate), and biological therapies (rituximab, abatacept and belimumab). For each recommendation, levels of evidence (mostly modest) and TF agreement (mostly very high) are provided. The 2019 EULAR recommendations are based on the evidence collected in the last 16 years in the management of primary 2002 SjS patients and on discussions between a large and broadly international TF. The recommendations synthesise current thinking on SjS treatment in a set of overarching principles and recommendations. We hope that the current recommendations will be broadly applied in clinical practice and/or serve as a template for national societies to develop local recommendations.